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Rheumatology (Oxford) 2019 Mar 18. Epub 2019 Mar 18.

Department of Internal Medicine, National Referral Center for Systemic and Autoimmune Diseases, Hôpital de la Pitié-Salpêtrière, Paris.

Objective: Orbital mass is a rare and sight-threatening manifestation of ANCA-associated vasculitides, which remains a therapeutic challenge. We aimed to describe the presentation, therapeutic management and outcome of ANCA-associated vasculitides-related orbital mass.

Methods: We conducted a French nationwide retrospective study of patients with orbital mass in the setting of ANCA-associated vasculitides according to ACR criteria and/or Chapel Hill Consensus Conference definitions. Read More

BMC Rheumatol 2019 8;3. Epub 2019 Mar 8.

2Department of Hematology, Skane University Hospital and Wallenberg Center for Molecular Medicine, Lund University, Lund, Sweden.

Background: Anti-neutrophil cytoplasmic antibodies associated vasculitides (AAV) are characterized by autoimmune small vessel inflammation. Eosinophils are multifunctional cells with both pro-inflammatory and immunoregulatory properties. Tissue activated eosinophils secrete cyto- and chemokines and form extracellular traps (EETs), they release free granules and produce reactive oxygen species. Read More

J Rheumatol 2019 Mar 1. Epub 2019 Mar 1.

From the Department of Internal Medicine, Department of Biochemistry, Department of Immunology & Department of Nephrology, CHU de Caen, Caen, F-14000, France; Department of Infectious Diseases, CH Mémorial,Saint-Lô, 50000, France. Address correspondence to Pr Achille AOUBA, Department of Internal Medicine and Clinical Immunology, CHU Côte de Nacre - Université Basse Normandie, Avenue de la Côte de Nacre, 14000 CAEN, FRANCE. Email:

Objective: Deficiency in alpha-1-antitrypsin (AAT) is a possible pathogenic cofactor in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). However, the clinical impact of AAT deficiency remains poorly established in this setting. This study aimed to describe the clinical phenotypes and outcomes of AAV according to AAT phenotypes. Read More

Rheumatology (Oxford) 2019 Feb 25. Epub 2019 Feb 25.

Department of Internal Medicine and Clinical Immunology, Normandie Univ, UNICAEN, CHU de Caen Normandie, Caen, France.

Objectives: In ANCA-associated vasculitis (AAV), classifications have emerged to individualize homogeneous clinical and outcomes patterns, including the recently defined anti-MPO granulomatosis with polyangiitis (GPA) subgroup. This study aimed to retrospectively evaluate the impacts of re-classification based on clinicopathological criteria and/or ANCA specificity.

Methods: A retrospective monocentric study conducted at Caen University Hospital led to the identification of PR3 or MPO-ANCA AAV patients from January 2000 or September 2011, respectively, to June 2016. Read More

Pol J Pathol 2018 ;69(4):384-387

It is well known that acute alveolar haemorrhage (AAH) is attributed to capillaritis in most cases with microscopic polyangiitis (MPA). In this article, we explore the cause of alveolar haemorrhage in MPA patients. In the present study, we extracted four autopsy cases of MPA with AAH. Read More

Respirol Case Rep 2019 Apr 5;7(3):e00407. Epub 2019 Feb 5.

Department of Thoracic Medicine The Prince Charles Hospital Brisbane Australia.

We describe a patient with underlying HIV presenting with progressive respiratory distress and acute renal failure. A unifying diagnosis of microscopic polyangiitis was made. Following immunosuppression induction with plasma exchange and intravenous corticosteroid and subsequent maintenance immunosuppression with intravenous cyclophosphamide in conjunction with renal replacement therapy he achieved remission. Read More

Eur J Case Rep Intern Med 2018 27;5(12):000978. Epub 2018 Dec 27.

Pulmonary Department, Loewenstein Lung Center, Löwenstein, Germany.

A crazy-paving pattern is a non-specific radiological sign which is characterized by the presence of diffuse ground-glass attenuation associated with interlobular septal thickening and intralobular lines. It was initially described as a pathognomonic sign of pulmonary alveolar proteinosis. However, it can be also found in many other diffuse acute and chronic lung diseases including diffuse alveolar haemorrhage (DAH), a rare and life-threatening clinical syndrome which can be caused by many conditions, the most frequent of these being capillaritis associated with systemic autoimmune diseases. Read More

Clin Rheumatol 2019 Feb 11. Epub 2019 Feb 11.

Rheumatology Unit, Internal Medicine Services, Hospital Italiano de Buenos Aires, Perón 4190, 1414, Buenos Aires, Argentina.

Background/objective: Our objective was to estimate incidence and prevalence rates of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) using data from a university hospital-based health management organization (HIMCO) in Latin America.

Methods: Multiple methods for case finding were used to ensure complete ascertainment. GPA was diagnosed if fulfilling ACR 1990 criteria or a clinical diagnosis was made by an experienced rheumatologist. Read More

Clin Rheumatol 2019 Feb 8. Epub 2019 Feb 8.

Jinling Clinical Medical College of Nanjing Medical University, Nanjing, China.

Objectives: To retrospectively investigate the clinical and histological features and outcomes of ANCA-associated glomerulonephritis (AAGN) with different ANCA serotypes.

Method: A total of 467 AAGN patients were divided into MPO-AAGN (MPO) and PR3-AAGN (PR3) groups according to ANCA serotype. Clinical and histological features and renal outcomes were compared. Read More

Case Rep Rheumatol 2019 6;2019:5013904. Epub 2019 Jan 6.

University of Tennessee Health Science Center, Division of Rheumatology, Memphis, TN, USA.

We report here the first case of systemic lupus erythematosus (SLE) and antineutrophil cytoplasmic antibody-associated vasculitis overlap syndrome (SLE/AAV) who had granulomatous polyangiitis (GPA) as the initial presentation. SLE/AAV overlap syndrome is an uncommon entity recently described in the literature. Prior reported patients with SLE/AAV overlap syndrome presented with SLE and microscopic polyangiitis (MPA). Read More

Ter Arkh 2018 Jun;90(6):55-58

I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia (Sechenov University), Moscow, Russia.

Aim: To evaluate clinical features and outcomes of renal involvement in patients with microscopic polyangiitis (MPA).

Materials And Methods: We enrolled 99 patients with MPA, diagnosed in accordance with the algorithm of the European Medicines Evaluation Agency (EMEA) and the Chapel Hill consensus conference definition (2012). Serum creatinine (sCr), estimated glomerular filtration rate (eGFR), hematuria and proteinuria were estimated. Read More

Ter Arkh 2018 May;90(5):13-22

V.A. Nasonova Scientific Research Institute of Rheumatology, Moscow, Russia.

Antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) are rare autoimmune disorders and characterized by severe multiple organ lesions with a potential fatal outcome. AAV comprises granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). Early diagnosis and treatment can significantly improve the AAV prognosis, but there are can be difficult, largely due to the lack of criteria for the classification MPA, whose main difference is the absence of granulomatous inflammation. Read More

Ter Arkh 2018 Apr;90(3):99-106

Pirogov Russian National Research Medical University, Moscow, Russia.

Pulmonary vasculitis is a group of rapidly progressing severe diseases characterized by vascular inflammation, destruction and necrosis of the pulmonary tissue. The pathological process in the lungs varies from diffuse alveolar hemorrhage to inflammation of the parenchyma, pleural effusion, thrombotic and thromboembolic complications. Depending on the size of the affected vessels, vasculites are divided into vasculites of large, medium and small vessels. Read More

Int J Rheum Dis 2019 Jan;22 Suppl 1:78-85

Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Vasculitis includes a group of disorders characterized by inflammation of the vessel wall and classified based on the diameter of the predominantly involved vessels. Granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis and Henoch-Schonlein purpura are the important entities in the small vessel vasculitis group, while polyarteritis nodosa and Kawasaki disease represent the medium vessel vasculitis group. The clinical manifestations may be non-specific and there may be considerable overlap with the other disorders. Read More

Front Neurol 2018 10;9:1166. Epub 2019 Jan 10.

Department of Neurology, Second Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China.

To provide a comprehensive review of the central nervous system (CNS) involvement in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), including the pathogenesis, clinical manifestations, ancillary investigations, differential diagnosis, and treatment. Particular emphasis is placed on the clinical spectrum and diagnostic testing of AAV. AAV is a pauci-immune small-vessel vasculitis characterized by neutrophil-mediated vasculitis and granulomatousis. Read More

Acta Otorhinolaryngol Ital 2018 Dec;38(6):517-527

Department and Clinic of Otolaryngology, Head and Neck Surgery, Wrocław Medical University Hospital, Borowska, Poland.

ENT manifestations are commonly observed in patients with small vessel vasculitis (SVV). The main aim of this study was to analyse and present the clinicopathological characteristics of individuals with SVV emphasising otorhinolaryngological symptoms. This study evaluated 64 patients, 41 with granulomatosis with polyangiitis (GPA) and 23 with microscopic polyangiitis (MPA). Read More

Korean J Intern Med 2019 Jan 9. Epub 2019 Jan 9.

Department of Rheumatology, Ome Municipal General Hospital, Tokyo, Japan.

Clinical trials and observational studies have established cyclophosphamide (CY) or rituximab plus glucocorticoid (GC) as standard remission induction therapies in patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA). However, because these regimens are associated with serious adverse drug reactions, the development of drugs with novel mechanisms of actions are needed. Progress in basic and clinical research has identified novel candidate targeting molecules, including B-cell activating factor (BAF), C5a receptor, and interleukin-6. Read More

Beijing Da Xue Xue Bao Yi Xue Ban 2018 Dec;50(6):1022-1026

Department of Rheumatology, Fouth Medical Center of PLA General Hospital, Beijing 100048, China.

Objective: To investigate the significance of a set of seven disease activities and extension measurements and their correlations between one and another for anti-neutrophil cytoplasmic autoantibody associated vasculitis (AAV).

Methods: A total of 121 patients from Peking University International Hospital and Fouth Medical Center of PLA General Hospital with confirmed diagnoses of AAV clinically were enrolled in the study, including 15 cases of eosinophilic granulomatous with polyangiitis (EGPA), 59 cases of granulomatous with polyangiitis (GPA) and 47 cases of microscopic polyangiitis (MPA). A hundred and twenty-one AAV patients were divided into death group and survival group according to their survival conditions. Read More

Yonsei Med J 2019 Jan;60(1):10-21

Division of Rheumatology, Department of Internal Medicine, and Institute for Immunology and Immunological Diseases, Yonsei University College of Medicine, Seoul, Korea.

Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is a group of systemic necrotising vasculitides, which often involve small vessels, and which lead to few or no immune deposits in affected organs. According to clinical manifestations and pathological features, AAV is classified into three variants: microscopic polyangiitis, granulomatosis with polyangiitis (GPA), and eosinophilic GPA. The American College of Rheumatology 1990 criteria contributed to the classification of AAV, although currently the algorithm suggested by the European Medicines Agency in 2007 and the Chapel Hill Consensus Conference Nomenclature of Vasculitides proposed in 2012 have encouraged physicians to classify AAV patients properly. Read More

Clin Rheumatol 2019 Apr 14;38(4):1147-1154. Epub 2018 Dec 14.

Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, 50-1 Yonsei-ro, Seodaemun-gu, Seoul, 03722, South Korea.

Objectives: We investigated whether elevated serum IgG4 at the time of diagnosis of microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) may be associated with concurrent IgG4-related disease (IgG4-RD) in immunosuppressive drug-naïve patients.

Methods: We retrospectively reviewed the medical records of 46 MPA and GPA patients with results on serum IgG4 and histology at diagnosis. Elevated serum IgG4 was defined as IgG4 > 135 mg/dL. Read More

Nat Rev Rheumatol 2019 Feb;15(2):91-101

Department of Medical Laboratory Science, Faculty of Health Sciences, Hokkaido University, Sapporo, Japan.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) affects systemic small vessels and is accompanied by the presence of ANCAs in the serum. This disease entity includes microscopic polyangiitis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and drug-induced AAV. Similar to other autoimmune diseases, AAV develops in patients with a predisposing genetic background who have been exposed to causative environmental factors. Read More

Int J Nephrol Renovasc Dis 2018 22;11:313-319. Epub 2018 Nov 22.

Department of Medicine, Division of Nephrology, UCLA David Geffen School of Medicine, Los Angeles, CA, USA,

Circulating antineutrophil cytoplasmic antibodies (ANCAs) are the central pathogenic mechanism for a group of systemic and renal syndromes called the ANCA-associated vasculitis (AAV). The nomenclature has changed from eponymous labeling to granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis. These syndromes predominantly affect the pulmonary and renal systems. Read More

Rheumatology (Oxford) 2018 Dec 7. Epub 2018 Dec 7.

Department of Paediatric Rheumatology, Alder Hey Children's Hospital, Liverpool, UK.

Objectives: The European initiative Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) aimed to optimize care for children with rheumatic diseases. Systemic vasculitides are very rare in children. Consequently, despite recent advances, paediatric-specific information is sparse. Read More

Best Pract Res Clin Rheumatol 2018 04 2;32(2):271-294. Epub 2018 Nov 2.

Department of Rheumatology, Hospital of Bruneck, Bruneck, Italy; Department of Rheumatology and Immunology, Medical University Graz, Graz, Austria.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and giant cell arteritis (GCA) are the most common primary systemic vasculitides of the adult population, while polymyalgia rheumatica (PMR) is a clinical syndrome often associated with GCA. Incidence and prevalence rates of AAV have been increasing in the last decades, whereas those of GCA and PMR have remained stable. The mutual interplay between environmental and genetic risk factors leading to the development of these diseases has been further analyzed in the last years. Read More

Best Pract Res Clin Rheumatol 2018 02 25;32(1):63-82. Epub 2018 Sep 25.

University Hospital Gasthuisberg, Herestraat 49, B3000, Leuven, Belgium. Electronic address:

Imaging plays an increasing role for confirming a suspected diagnosis of giant cell arteritis (GCA) or Takayasu arteritis (TAK). Ultrasound, magnetic resonance imaging (MRI), and computed tomography demonstrate a homogeneous, most commonly concentric, arterial wall thickening. 18F-Fluorodeoxyglucose (FDG) positron emission tomography (PET) displays increased FDG uptake of inflamed artery walls delineating increased metabolism. Read More

J Rheumatol 2018 Dec;45(12):1705

Department of Rheumatology and Clinical Immunology, Kobe University Hospital, Kobe, Japan.

Drugs Aging 2019 01;36(1):53-63

Rheumatology Department, Policlinico S. Matteo, Fondazione IRCCS, University of Pavia, Pavia, Italy.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) usually affect adults and older populations. The well recognized clinical (i.e. Read More

Clin Rheumatol 2019 Apr 27;38(4):1039-1046. Epub 2018 Nov 27.

Department of Nephrology Center, Toranomon Hospital, 2-2-2, Toranomon, Minatoku, 105-8470, Japan.

Objectives: Hypertrophic pachymeningitis (HP) is a rare complication in patients with anti-neutrophil antibody-associated vasculitis (AAV); its clinical features, incidence, and risk factors remain unknown. We aimed to clarify the prevalence, clinical features, and factors associated with new-onset HP in patients with AAV.

Method: A retrospective cohort study involving 93 patients with AAV was conducted. Read More

BMC Pulm Med 2018 Nov 19;18(1):170. Epub 2018 Nov 19.

Centre for Experimental Medicine, Queen's University Belfast, 97 Lisburn Road, Belfast, BT9 7BL, Northern Ireland.

Background: Non-Tuberculous Mycobacterial-pulmonary disease (NTM-PD) is increasing in incidence and prevalence. Mycobacterium abscessus (M.abscessus) is a rapid growing multi-resistant NTM associated with severe NTM-PD requiring prolonged antibiotic therapy. Read More

J Thorac Dis 2018 Sep;10(9):5560-5575

Interdisciplinary Research Group in Pneumology, Institute of Sanitary Research of Santiago de Compostela (IDIS), Santiago de Compostela, Spain.

Systemic vasculitides frequently affect the pulmonary vasculature. As the signs and symptoms of pulmonary vasculitis are variable and nonspecific, diagnosis and treatment represent a real challenge. Vasculitides should be given consideration, as these diseases present severe manifestations of rapidly progressing pulmonary disease. Read More

Int J Nephrol 2018 16;2018:6369814. Epub 2018 Oct 16.

Abdomen Center, Nephrology, Helsinki University Hospital, Helsinki, Finland.

Aim: We describe the clinical pattern of ANCA-associated vasculitis (AAV) and assess long-term prognostic factors of patients and renal survival and relapse.

Methods: Data from 85 patients with renal biopsy-proven AAV at a single center with up to 20-year [median 16.2 years (95% CI 14. Read More

Semin Respir Crit Care Med 2018 08 7;39(4):482-493. Epub 2018 Nov 7.

Department of Respiratory Medicine, Hospices Civils de Lyon, National Reference Center for Rare Pulmonary Diseases, Lyon, France.

Defined by the accumulation of red blood cells into the alveolar space, diffuse alveolar hemorrhage (DAH) is a severe and potentially fatal medical condition requiring careful attention. In contrast to simple extravasation of erythrocytes facilitated by impaired hemostasis or hemodynamic causes, DAH in vasculitis is due to capillaritis, that is, inflammation of capillaries. Dyspnea, hemoptysis, chest infiltrates, and abrupt fall of blood hemoglobin level represent the cardinal features of DAH; yet, hemoptysis is lacking in one-third of cases. Read More

Semin Respir Crit Care Med 2018 08 7;39(4):465-470. Epub 2018 Nov 7.

Assistance Publique Hôpitaux de Paris, Bichat Hospital, Rare Pulmonary Diseases Reference Center, Paris, France.

Pulmonary fibrosis is observed in a substantial number of patients with ANCA-associated vasculitis (AAV), 15% in a recent German series, and may be more frequent in Asian populations. ANCA are usually of anti-MPO specificity and microscopic polyangiitis is the most frequent vasculitis. Pulmonary fibrosis may increase the risk of death in patients with AAV. Read More

Semin Respir Crit Care Med 2018 08 7;39(4):459-464. Epub 2018 Nov 7.

Department of Nephrology, Hôpital Européen Georges Pompidou, Assistance Publique- Hôpitaux de Paris, Paris France.

Microscopic polyangiitis (MPA) is one of the main clinical presentations of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Although the disease is defined by clinical and pathological criteria, the anti-myeloperoxidase (MPO) specificity of ANCAs is observed in almost 80% of MPA patients. The direct pathogenic role of anti-MPO antibodies has been proven in animal models, in which the disease was transmitted by transfer of anti-MPO antibodies or anti-MPO-specific splenocytes. Read More

Semin Respir Crit Care Med 2018 08 7;39(4):425-433. Epub 2018 Nov 7.

Department of Pathology and Laboratory Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California.

Vasculitides are a heterogeneous group of disorders in which inflammation of blood vessel walls is present at least some time during the course of the disease. Vasculitides can affect any caliber or type of vessel in many anatomic sites; however, the disease can alter more than just vasculature. Given the diversity of vasculitides, in 2012, a revised classification system was proposed to categorize vasculitides by the type of vessel involved including size, function, and structural attributes. Read More

Semin Respir Crit Care Med 2018 08 7;39(4):413-424. Epub 2018 Nov 7.

University of North Carolina Kidney Center, Chapel Hill, North Carolina.

Antineutrophil cytoplasmic antibodies (ANCAs) are autoantibodies specific for antigens located in the cytoplasmic granules of neutrophils and lysosomes of monocytes. ANCAs are associated with a spectrum of necrotizing vasculitis that includes granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis. Pulmonary vasculitis and related extravascular inflammation and fibrosis are frequent components of ANCA vasculitis. Read More

MBio 2018 10 30;9(5). Epub 2018 Oct 30.

Office of the Chief Scientists, U.S. Army Medical Research Institute of Infectious Diseases, Fort Detrick, Frederick, Maryland, USA

Lassa virus (LASV) causes a severe, often fatal hemorrhagic disease in regions in Africa where the disease is endemic, and approximately 30% of patients develop sudden-onset sensorineural hearing loss after recovering from acute disease. The causal mechanism of hearing loss in LASV-infected patients remains elusive. Here, we report findings after closely examining the chronic disease experienced by surviving macaques assigned to LASV exposure control groups in two different studies. Read More

J Allergy Clin Immunol Pract 2019 Mar 24;7(3):1004-1009. Epub 2018 Oct 24.

Department of Internal Medicine, Division of Nephrology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands. Electronic address:

Background: Azathioprine hypersensitivity syndrome is a rare complication of azathioprine therapy. Its symptoms resemble infection or relapse of inflammatory disease, hindering correct diagnosis. Current literature is limited to sporadic case reports and reviews. Read More

Z Rheumatol 2018 Dec;77(10):907-922

Abt. für Rheumatologie, Immunologie und Rehabilitation, Kantonsspital St. Gallen, St. Gallen, Schweiz.

Eosinophilic granulocytes form peripheral effector cells controlled by Th2 lymphocytes, which cause local cell, tissue, and functional disorders of infiltrated organs via the release of cytotoxic basic proteins and oxygen radicals. Diseases associated with eosinophilia include systemic and organ-related forms. The lungs are involved in eosinophilic granulomatosis with polyangiitis (EGPA, formerly known as Churg-Strauss syndrome), acute and chronic eosinophilic pneumonia, as well as in an organ manifestation in hypereosinophilic syndrome and certain parasitic diseases. Read More

Int J Rheum Dis 2018 Sep;21(9):1734-1740

Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

Aim: Antineutrophil cytoplasmic antibody-associated vasculitides (AAV) are a group of small vessel vasculitis with systemic presentations and considerable morbidity and mortality. Pregnancy in these patients poses a significant therapeutic challenge. There is limited published literature regarding pregnancy in AAV. Read More

Explore (NY) 2018 11 2;14(6):430-434. Epub 2018 Aug 2.

Institute of Life Sciences, Chongqing Medical University, Chongqing 400016, China. Electronic address:

Microscopic polyangiitis (MPA) is a systemic disease described as necrotizing vasculitis that affects capillaries, venules and arterioles. Hormone-based immunosuppression therapy is the common treatment, but with severe side effects. Hence, non-hormone or low-hormone therapies may be another option. Read More

Int J Rheum Dis 2019 Jan 18;22 Suppl 1:90-94. Epub 2018 Oct 18.

Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

The incidence and clinical features of antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) have been shown to vary according to geographical areas, with granulomatosis with polyangiitis (GPA) being more common in northern Europe and microscopic polyangiitis (MPA) being more common in Asian countries. The annual incidence of GPA among Asians varies between 0.37/million to 2. Read More

Clin Kidney J 2018 Oct 15;11(5):659-666. Epub 2018 Mar 15.

Division of Nephrology, Department of Medicine, Queen's University, Kingston, ON, Canada.

Background: antineutrophil cytoplasmic antibody-associated vasculitis typically arises in post-reproductive years, but can occur during pregnancy. Concerns of treatment-related teratogenicity persist, while efficacy and safety of new therapies including intravenous immunoglobulin (IVIG) and rituximab are uncertain. There remains a paucity of maternal, fetal and pregnancy outcome data in these women, and therefore a lack of guidance on safe treatment for clinicians. Read More

Postgrad Med J 2018 Dec 3;94(1118):728. Epub 2018 Oct 3.

Department of Internal Medicine, University Hospital of Heraklion, Heraklion, Greece.

Rheum Dis Clin North Am 2018 11 7;44(4):525-543. Epub 2018 Sep 7.

Division of Nephrology, Vasculitis and Glomerulonephritis Center, Massachusetts General Hospital, 101 Merrimac Street, Boston, MA 02114, USA. Electronic address:

Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is the most common cause of rapidly progressive glomerulonephritis. ANCAs play an important role in the pathogenesis and diagnosis of AAV. The classic renal lesion in AAV is a pauci-immune necrotizing and crescentic glomerulonephritis. Read More

J Autoimmun 2019 Jan 17;96:134-141. Epub 2018 Sep 17.

Université de Toulouse Paul Sabatier, Toulouse, France; Service de Médecine Interne, CHU Toulouse Purpan, Toulouse, France.

Objective: The aim of our study was to assess major cardiovascular event incidence, predictors, and mortality in ANCA-associated vasculitis (AAV).

Methods: We conducted a retrospective cohort study of all GPA or MPA, according to Chapel Hill Consensus Conference classification criteria, diagnosed between 1981 and 2015. Major cardiovascular event was defined as acute coronary artery disease, or ischemic stroke, or peripheral vascular disease requiring a revascularization procedure. Read More

Clin Rheumatol 2019 Feb 15;38(2):487-494. Epub 2018 Sep 15.

Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Republic of Korea.

We investigated whether albumin to globulin ratio (AGR) at diagnosis may be associated with all-cause mortality in immunosuppressive drug-naïve patients with microscopic polyangiitis (MPA). We retrospectively reviewed the medical records of 88 MPA patients, who were first classified and in whom medications was first initiated in our tertiary Hospital. We collected clinical and laboratory data as well as the rate of all-cause mortality. Read More